MOC-PS(SM) CME article: management considerations in the treatment of craniosynostosis

After studying this article, the participant should be able to: 1. Recognize the anatomical characteristics of the individual forms of craniosynostosis. 2. Differentiate deformational plagiocephaly from craniosynostosis. 3. Identify the pros and cons of individual types of craniosynostosis operations (i.e., endoscopic craniectomy and open cranioplasty procedures). 4. Understand risks of operative procedure versus the natural history of untreated craniosynostosis.

The term "craniosynostosis" refers to the premature fusion of one or more cranial vault sutures and its associated skull deformities. The pattern of skull deformities for each of the sutures (metopic, coronal, sagittal, and lambdoid) is characteristic from patient to patient, although variable in the degree of severity. The skull pattern is predictable in that restriction of growth occurs in a plane perpendicular to the plane of the fused vault suture, and compensatory changes occur frequently, parallel to it. When skull deformities are recognized with midfacial and extremity deformities, often the craniosynostosis is referred to as syndromic, implying a genetic basis for the skeletal maldevelopment. Indications for surgery relate to benefits in changing the shape of the skull toward normal and potentially avoiding brain maldevelopment. Brain injury is presumed to be related to local or regional increases in intracranial pressure. A broad range of surgical options to treat craniosynostosis exist, from strip craniectomy to comprehensive, or whole vault, cranioplasty. The optimal surgical timing for these approaches must balance both the desire for early intervention to reduce the effects of bone restriction on brain growth and the ability of a child to withstand the rigors of surgery. Complications of surgery include blood loss, bone defects, and scalp scarring. The most common, significant, postoperative complication, however, is incomplete correction of the skull deformity. Infection in nonsyndromic synostosis surgery is rare. Team management and longitudinal follow-through will aid in determining efficacy and safety of the available surgical approaches.